This document discusses various diseases and conditions of the orbit, including proptosis, Grave's ophthalmopathy, enophthalmos, developmental anomalies, orbital inflammations, and more. It defines key terms, describes causes, clinical features, treatment, and related pathologies for each topic. Images and diagrams are included to illustrate some conditions like craniosynostosis and its types.

1. Disease of the
Orbit
Presented By
Shagufta Kadri
2nd Year B.SC Optometry
Aditya jyot Institute of Optometry
aditya Jyot
Eye Hospital Pvt Ltd
ISO 9001 : 2008

2. Proptosis

3. Proptosis
• Define: It is define as the forward displacement
of eyeball beyond the orbital margin
• Causes: Unilateral Proptosis
• Bilateral Proptosis
• Pulsating Proptosis

4. Proptosis
Unilateral Proptosis (CT, VICTM)
Congenital: dermoid cyst and oribtal tertoma
Traumatic: orbital haemorrhage,traumatic aneurysm
and emphysema.
Vascular lesion: angioneurotic oedema, orbital
aneurysms.
Inflammatory lesion: orbital cellulitis, abscess,
tuberculoma.
Cyst: haematic cyst, implantation and parasitic cyst.
Tumor: Metastatic
Mucoceles: paranasal sinus.

6. Proptosis
Bilateral Proptosis (SIT, BED)
Systemic disease: histiocytosis and amyloidosis
Inflammatory: Mikulicz’s syndrome
Tumors: lymphoma, neuroblastoma and leukemic
infiltration
Bones: osteitis deformans, rickets and acromegaly
Endocrinal: thyrotoxic and thyrotropic.
Developmental: oxycephaly .

8. Proptosis
Pulsating proptosis
It is caused by pulsating vascular lesions such
as Carotico cavernous fistula and saccular
aneurysm
These include Congenital meningocele and
deficient orbital roof.

9. Pulsating Proptosis

11. Grave’s Opthalmopathy
Define: Grave’s opthalmopathy also known as
(Thyroid eye disease TED) is an autoimmune
inflammatory disorder of the orbit which include
Lid Lag + Lid retraction + Proptosis.

12. Grave’s Opthalmopathy
Pathogenesis
Inflammation of EOM which is characterized by
pleomorphic cellular infiltration associated with
increased infiltration and osmotic imbibition.
The muscle become enlarged sometimes upto 8 times
their normal size and may compress the optic nerve.
Inflammatory cellular infiltration with
lymphocytes,plasma, cell marophages and mast cells of
interstitial tissues, orbital fat and lacrimal gland
associated with GAGs and retention of fluid.

13. Grave’s Opthalmopathy
Clinical features
Lid Signs:
Stellwag’s sign: Infrequent blinking Staring
Dalrymple’s sign: Lid retraction Staring and
Frightened appearance
Enroth’s sign: Fullness of eyelid Puffy oedema
Von Graefe’s sign: Globe downward Lid lags behind

14. Grave’s Opthalmopathy
• American Thyroid Association Classification
[NOSPECS]
Class 0: No signs and symptoms
Class 1: Only signs, no symptoms
Class 2: Soft tissue involvement with signs and symptoms
Including lacrimation, photophobia
Class 3: Proptosis is well established
Class 4: EOM involvement
Class 5: Cornea involvement
Class 6: Sight loss

15. Grave’s Opthalmopathy
Treatment: Non Surgical treatment
Head elevation at night, cold compression at morning
Lubricating artificial tear drop,ointment at bed time.
EOM surgery
Eyelid surgery

17. Enopthalmos
Define : It is the posterior displacement of the eyeball
within the orbit due to changes in the volume of the
orbit relative to its content or loss of function of the
orbitalis muscle.

18. Enopthalmos
Etiology
Congenital: Micropthalmos and maxillary hypoplasia
Traumatic: Blow out fractures of floor of the orbit
Post-inflammtory: Cicatrization of EOM
Paralytic Enopthalmos: It is seen in Horner’s syndrome
Atrophy of orbital contents: Senile Atrophy of orbital fat
Atrophy due to irridiation of malignant tumor

20. Developmental Anamolies of the Orbit

21. Developmental Anamolies of the Orbit
Developmental anomalies of the orbit are commonly
associated with abnormalities of skull and facial bones.
Ocular features of developmental orbital anamolies may
be one or more of the following:
Proptosis
Papilloedema
Strabismus and
Optic atrophy

22. Developmental Anamolies of the Orbit
Anamoly
Brachycephaly
Oxycephaly
Scophocephaly
Trigonocephaly
Sutures closed prematurely
All cranial sutures
Coronal sutures
Sagittal sutures
Frontal sutures

24. Developmental Anamolies of the Orbit
Craniosynostosis
Define: It is a condition in which one or more of the fibrous
sutures in an infant skull prematurely fuses.
Etiology:
Non Syndromic craniosynostosis: It is the most common
type of craniosynostosis and its cause its unknown although
its thought to be combination of genes and environmental
factors
Syndromics Craniosynostosis: It is caused by certain genetic
syndrome such as Apert syndrome, Crouzon syndrome
which can affect your baby’s skull development.

25. Craniosynostosis
• Types of Craniosynostosis
Metopic Synostosis: It occurs when there is a fusion
between sagittal strucrure and nose. Metopic synostosis
results in triangular development of scalp.
Lamdoid Synostosis: It is the rarest types of
craniosynostosis and affects the back portion of the
head.Lamdoid synostosis results in twisted shape of skull.

26. Craniosynostosis
Coronal craniosynostosis: It occurs when there is an
early fusion between the coronal structures of the skull,
which affects the higher eye socket and results in the flat
development of the forehead.

27. Metopic
craniosynostosis

30. Craniosynostosis
Etiology
Doctors do not know the exact cause of craniosynostosis.
Genetic mutation is stated one of the most possible
causes of craniosynostosis.
Genetic mutation leads to the defects in cell sutures that
cause them to fuse too early
Another possible cause of craniosynostosis is the
irregular position of the baby in the womb

31. Craniosynostosis
Symptoms
Abnormal shape of head
Early fusion of head
Learning disabilities
Frequent headaches

32. Crouzon’s Syndrome

33. Crouzon’s Syndrome
Define: Craniofacial dysostosis (crouzon’s syndrome) is a
genetic disorder characterized by premature closures of
certain sutures.

34. Crouzon’s Syndrome
Clinical features
Proptosis due to shallow orbits
Divergent squint
Hypertelorism i.e, widely separated eyeballs
Dental problems, vision loss and narrow ear canals.
In some cases, there is opening of lip and roof of mouth
High arched plate
Hooked (parrot beak) nose

36. Treacher Collin Syndrome
Define: Mandibulofacial dysostosis refers to a condition
resulting from hypoplasia of zygoma and mandible.
It is a genetic disorder characterized by deformities of
the ears, cheeekbones, eyes and chin.

37. Treacher Collin Syndrome
Clinical features
Indistinct inferior orbital margin
Coloboma of the lower eyelid
External ear deformity
Bird like face
Complications: Breathing problem, Vision problem,
Hearing problrem, Cleft palate.
Those affected generally have a normal intelligence

38. Treacher Collins Syndrome
Prognosis: Generally normal life expectancy.
Symptoms: Downward slanting eyes, small jaw and chin,
alters bones and tissues.
Causes: Genetic
Treatment: Reconstructive surgery, hearing aids, Speech
therapy

40. Oxycephaly Syndactyle
Define: Apert Syndrome is a genetic disorder characterized
by the premature fusion of skull bones.
The early fusion prevents the skull from growing
normally and affects the shape of the head and face.
In addition,a varied number of fingers and toes are fused
together
Bulging and wide set eye, a beaked nose and
underdeveloped leading to crowded teeth.

41. Oxycephaly Syndactyle
Additional signs and symptoms: Hearing loss, unusually
heavy sweating, oily skin with severe acne,patches of
missing hair, short height.
Treatment: Surgery is performed to correct abnormal
bone growth

43. Hypertelorism
It is a condition of widely separated eyeballs resulting
from widely separated orbits and broad nasal bridge.
In this condition the distance between the inner eye
corners as well as the distance between the pupils is
greater than normal.
Hypertelorism may occur as a part of various syndromes
such as Apert’s Syndromes

44. Hypertelorism
Embryology and Pathology
It probably get started at about 28mm embryo stage
defective development of the nasal capsule.
Early ossification of the lesser wings of sphenoid leads to
arrest of the orbits in the foetal position.
Deficient lateral movement of the orbit.

45. Hypertelorism
Clinical Features
Increased IPD may be 85mm normally
Divergent squint
Antimogoloid slant
Optic Atrophy due to narrow optic canal in some cases
Treatment: Surgery (Facial bipartition, Box osteotomy)

47. Orbital
Inflammations

48. Preseptal Cellulitis
Define: Preseptal (or periorbital) refers to infection of the
subcutaneous tissues anterior to the orbit septum
Causative organism: Staphylococcus aures or
streptococcus pyogenes.

49. Preseptal Cellulitis
Modes of Infection:
Exogenous infections may results following skin
laceration or insect bites.
Extension from local infections such as from an acute
hardoelum or acute dacryocystitis.
Endogenous infection may occur by haematogenous
spread from remote infection of the middle ear or upper
respiratory tract.

50. Preseptal Cellulitis
Clinical features
Preseptal cellulitis present as inflammatory oedema of
the eye lids and periobital skin with no involvement of
the orbit.
Painful acute periorbital swelling,erythema and
hyperaemia of the lids.
There may be associates fever and leukocytosis.

51. Preseptal Cellulitis
Signs and symptoms: Pain on eye movement
 limited eye movement
Swelling
Redness
Discharge
Blurred Vn
Treament: Oral antibiotics and anti inflammatory drug.

53. Orbital Cellulitis
Define: Orbital cellulitis refers to an acute infection of the soft
tissues of the orbit behind the orbital septum.

54. Orbital Cellulitis
Etiology
Exogenous Infection: It may result from penetrating
injuries especially when associated with retention of
intraorbital foreign body and following operations like
orbitotomy, dacryocystectomy.
Extension of Infection: It occurs from neighbouring
structures which include paranasal sinuses, teeth,face,lid
intracranial cavity and intraorbital structures.

55. Orbital Cellulitis
Endogenous Infection: It may rarely develop as
metastatic infection from breast abscess, puerperal
sepsis, thrombophlebitis of legs.
Causative organism: Those commonly involved are
Streptococcus pyogenes, Staphylococcus aureus,
Haemophilus Influenzae.

56. Orbital Cellulitis
Symptoms: It includes severe pain and swelling which is
increased by movements of eye or pressure
Other symptoms may include fever, nausea, vomitting ,
prostrations and sometimes loss of vision.
Signs: Swelling of Lids by woody hardness and redness.
Chemosis of conjuctiva, which may protrude and become
dessicated or necrotic.
Eyeball is proptosed axially.
There is mild to severe restrictions of ocular movements

58. Orbital Mucormycosis
Define:Mucormycosis is a rare fungal infection of the orbit
caused by mold of the order mucorales.
Infections usually begins in the sinuses and erodes into
the orbital cavity
It is characterized as Rhino-orbital, Rhino-
cerebral,Rhinonasal,pulmonary,cutaneous and
gastrointestinal infections.
These organism have a tendency to invade vessels and
cause ischemic necrosis.

59. Orbital Mucormycosis
Clinical Features
The patients prone to such infections are diabetes and
immuno-compromised such as those with Renal
failure,Malignant tumors or those on antimetabolite or
steriod therapy .
Necrotic areas with black eschar formation may be seen
on the mucosa of the palate, turbinates and nasal
septum and skin of eyelids

60. Orbital Mucormycosis
Complications: If not treated energetically, patient
develops meningitis,brain abscess and dies within days to
week.
Treatment: Is often difficult and inadequate, therefore
recurrences are common.
Antifungal drug
Surgery

62. Orbital Periostitis
Define: Orbital Periostitis i.e, inflammation of the
periorbital.It is rare but particularly affects the orbital
margin.
It is more often due to injuries,extension of inflammation
from surrounding structures.
Tubercular periostitis is known in children and Syphilitic
in adults.
In traumatic cases it is the margin that is naturally most
affected.

63. Orbital Periostitis
Clinical features
Anterior orbital periostitis: It involves the orbital margin
and is characterized by severe pain,tenderness and
swelling.
Subperiosteal abscess.
Posterior orbital periostitis:It is characterized by deep
seated orbital pain,mild to moderate proptosis.
When orbital apex is implicated various ocular palsies
may develop and Amaurosis due to involvement of optic
nerve.

64. Orbital Periostitis
Treatment: Antibiotic drugs
In deep seated periostitis surgery may be necessary and
should not be too delayed.

66. Cavernous Sinus Thrombosis

67. Cavernous Sinus Thrombosis
Define: CST is the formation of the blood clot within the
cavernous sinus

68. Cavernous Sinus Thrombosis
Etiology
Bacterial Infection in another part of the skull or face can
spread to cavernous sinus (Staphylococcus aureus,
Streptococcus pneumonia)
 Fungal infection are less common – Aspergillus and
Rhizopus Species.
CST most commonly results from contagious spread of
infection from PNS and dental infection.

69. Cavernous Sinus Thrombosis
Communication and source of infection
Anteriorly the superior and inferior opthalmic vein drains in
the sinus.Therefore, infection to Cavernous Sinus may spread
from infected facial wounds,orbital cellulitis and sinusitis
Posteriorly Labyrinthine veins brings infections from the
middle ear.
Superiorly, the cavernous sinus communicates with veins of
the cerebrum and may be infected from meningitits and
cerebral abscess.

70. Cavernous Sinus Thrombosis
Inferiorly, the sinus communicates with pterygoid venous
plexus.
Medially the two cavernous sinuses are connected with
each other by transverse sinuses which account for
transfer of infection from one side to other.
Signs and Symptoms: Severe pain in the eye and
forehead on the affected side.
Proptosis develops rapidly..
Palsy of 3rd 4th and 6th cranial nerves occurs frequently.

71. Cavernous Sinus Thrombosis
Treatment: Antibiotics drugs.
Analgesic Drugs, Anti inflammatory, Analgesic Drugsand
Antocoagulants.

73. Idiopathic Orbital Inflammatory
Disease
Idiopathic Orbit inflammatory disease also known as
(Pseudotumor)
It produces Proptosis due to non neoplastic mass in the
orbit.
 It can occur throughout the orbit from the region of
lacrimal gland to the orbital apex.
It can occur at any age but commonest is 40 and 60 years
and slightly commoner in man.

74. Idiopathic Orbital Inflammatory
Disease
Clinical features
Swelling or puffiness of eyelids,proptosis,orbital pain,
restrcited eye movements,diplopia, chemosis and redness.
Spontaneous remissions after a few weeks are known in
pseudotumors.
In some patients prolonged inflammation may cause
progressive fibrosis of the orbital tissue.
Treatment: Course of systemic corticosteroid and
Radiotherapy

76. Orbital tumors

77. Developmental tumors
Dermoids: A dermoid is an overgrowth of normal non
cancerous tissue in an abnormal condition.
It is of two types (a)Simple dermoid: it is seen in infancy.
Appear as a firm, round,localised lesion in the upper
temporal or upper nasal aspect of the orbit
These are located anterior to the orbital septum.

78. Developmental tumors
(b)Complicated Dermoid: These are present in adoloscence
with proptosis or a mass lesion.They may be associated
with bony defects.
Epidermoid: It is composed of epidermis without any
epidermal appendages in the wall of the cyst.the cyst
wall contain Keratin debris.
Lipodermoids: These are solid tumors usually seen
beneath the conjuctivaThese are mostly located adjacent
to the superior temporal quadrant of the globe.

79. Developmental tumors
• Teratomas: These are composed of ectoderm,mesoderm
• And endoderm.
• These may be solid,cystic or a mixture of both
• Most of these are benign but some solid tumors in
newborns are malignant
• Cystic tumors may b e excised without removing the eye
ball.

81. Vascular Tumors
These are the most common primarys Benign tumors of
the orbit.These can be either Haemangiomas or
Lymphangiomas.
Capillary Haemangiomas: It is a (strawberry birthmark)
consisting of an abnormal overgrowth of tiny blood
vessels.
Eye involvement includes eyelids, conjuctiva and orbit.
This tumor is consist of varying size small vascular
channels without true encapsulation.
It is most common in infants.

82. Vascular Tumors
Signs: Superficial cutaneous lesions are bright red.
A large tumor may enlarge and change in color to a deep
blue during crying or straining
Treatment: These tumors usually do not require any
treatment.
In severe case radiations and Cryotherapy is given.

84. Cavernous Haemangioma: Also called as Cerebral
cavernous malformaton (CCM).It is a type of blood vessel
malformation where a collection of dialated blood
vessels form a benign tumor.
Because of these malformations blood
flow through the cavities or caverns is slow.
Signs: Axial proptosis which may be associated with optic
disc oedema
 A lesion at the orbital apex may compress the optic
nerve without causing proptosis
Vascular Tumors

85. Treatment: Surgical excision of the tumor is undertaken
Sclerotherapy
Steroid drugs.
Vascular Tumors

86. Optic Nerve Tumor
Optic nerve Glioama: It is a benign tumor arising from the
astrocytes.
It is a slow growing brain tumor that arises from the optic
chiasma.
As the tumor progresses,it presses on the optic nerve
causing a child’s vision to worsen.
Clinical features: Early Vn loss
Unilateral proptosis
Fundus examination may show optic atrophy,
Papilloedema,Venous enlargement.

87. Treatment: Radiotherapy
Chemotherapy for tumor intracranial extension.
 Dana Farber/Boston:One of the world’s largest pedriatic
Glioma programs is carried out here.
They have an extensive expertise for treating all types of
Glioma including optic nerve glioma.
Optic Nerve Tumor

89. Rhabdomyosarcoma
Define:It is a type of sarcoma of the orbit arising from
Extra ocular muscle.
Most common soft tissue in children
Parental use of Cocaine and Marijuana
Birthing order and accelerated in Utero growth.
Signs and symptoms: Tingling sensation,numbness,Pain
and movement may be affected
Childhood sign of rhabdomyosarcoma is swelling or
lumps that keep getting bigger.

90. Rhabdomyosarcoma
Bleeding from nose, vagina, rectum or throat.
Treatment: High dose Radiation therapy.
Combined with systemic Chemotherapy is very effective.

91. Lymphomas
 Define: Lymphoma is a type of cancer of lymphatic
system.
This type of cancer starts in Lymphocytes as it is present.
As it is present in blood stream it can spread or
metastatize to different parts of the body.
These can be classified into two distinct groups:
Hodgkin’s Lymphoms (HL) and non Hodgkin’s
Lymphomas (NHL)
These may involve orbit,lacrimal glands and
subconjuctival tissue.

92. Lymphomas
Signs and Symptoms:It is similar to those in illness such
as viral fever and commom cold.
Swelling are normally painless,but pain may occur if
enlarges glands press on organs, bones and other
structures.
Night sweats, fever and chills, weight loss.
Treatment: Antibody therapy, Radiation therapy,
Chemotherapy,Steroids
Surgery.

94. Multiple Choice Question
Q1) Apert Syndrome is also known as
A) Mandibulofacial dysostosis
B) Oxycephaly dysostosis
C) Craniofacial dysostosis
D) Craniosynostosis

95. Multiple Choice Question
Q2) World’s largest Dana Farber Boston programme is
carried out for which tumor of eye?
A) Lymphomas
B) Rhabdomyosarcoma
C) Cavernous Haemongioma
D) Optic nerve Glioma

96. Multiple Choice Question
Q3) In which Syndrome IPD is greater than Normal?
A) Apert Syndrome
B) Crouzon’s Syndrome
C) Hypertelorism
D) Marfan’s Syndrome

97. Multiple Choice Question
Q4) Guess the Disease??
A) Cavernous Sinus thrombosis
B) Capillary haemongioma
C) Cavernous haemongioma
D) Lymphomas

98. Multiple Choice Question
Q5) Where does Preseptal Cellulitis occurs?
A) Anterior Septum
B) Posterior Septum
C) Optic canal
D) Lesser wing of sphenoid

99. Thank u
For allowing me to speak and
listening to my Presentation.